Life is not particularly easy for Megan Thomson, who, among other things, is chronically hungry.

The four-year-old Burnaby girl has Prader-Willi Syndrome (PWS), a relatively rare genetic disorder that produces a feeling of overwhelming hunger that can lead to obesity and even death from binge eating.

It also affects many parts of the body, causing low muscle tone, short stature, cognitive disabilities, and problem behaviours along with the eating disorder.

“She’ll be eating and still have an empty feeling,” said mother Cindy Thomson of her daughter, whose story will be among many featured during the 47th annual Variety Show of Hearts Telethon on Saturday and Sunday on Global TV.

“It requires 24/7 supervision, and she’ll be on a calorie-restricted diet for life. They don’t burn as many calories as typical children and tire a lot easier.”

Thomson said it is a struggle at family get-togethers or overnight visits with people who often do not realize the importance of keeping food locked away.

“We avoid a lot of functions where there’s food involved. If you’re hungry all the time, you try to fill that emptiness inside.”

She said crowded places like shopping malls are also difficult because Megan gets stressed with too many people around.

Because of her low muscle tone in her torso and limbs, Megan couldn’t walk independently when she was younger.

So Variety provided funding for a Rifton Gait Trainer walker, which Thomson — in a wheelchair herself because of MS — says has worked miracles for her daughter.

“She’s had it for one-and-a-half years, and she’s walking independently now,” said Thomson, adding that she and husband Glen couldn’t have afforded the special walker on their own.

Megan’s doctor, Jean-Pierre Chanoine, a clinical professor and head of endocrinology and diabetes at BC Children’s Hospital, said three to four children are born with PWS each year in B.C.

He said life expectancy is less than normal — “They can expect to live into their 50s, but it’s getting better all the time” — and there has been a lot of progress over the past 20 years, although children suffer from low muscle tone and can quickly put on weight by overeating.

“The main factor (in treatment) is followup and control at home,” Chanoine added. “Patients have to be kept busy, and it’s a difficult job for parents. But today, parents are much more aware and keep their children very active and (are good) at hiding food.”

Chanoine noted that children with PWS are very quiet and unaggressive, but also have a moderate developmental delay, which can be reflected in difficulty learning. “One characteristic is they don’t go through puberty spontaneously. So, it has to be induced (with hormones).”

Also highlighted during this year’s telethon is 16-year-old Stephanie Cruz Parada, who was diagnosed with congenital cataracts at eight months of age and has chronic liver disease and Sjogren’s Syndrome — an autoimmune disease in which her white blood cells attack her moisture-producing gland, causing dysfunction of the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas and the central nervous system.

Stephanie’s mother, Sandra Parada, said that Variety has provided funding for medications and most recently a bursary to attend the Mediated Learning Academy, which she said gives her daughter “the opportunity to complete her studies in an environment of tranquillity, equality and comfort.”

Stephanie must take powerful drugs, which can have strong side effects.

“She’s in treatment all the time and gets very sick, very often,” added Parada. “And she can catch anything. We have to be very, very careful.

“She can only go to school infrequently. She only went two days this week.”

Parada noted there’s no cure, and Stephanie also has an enlarged heart with a cardiac defibrillator in her chest.

This year’s Variety telethon will feature a live performance by Canada’s own Chantal Kreviazuk, who launches the event, as well as pre-recorded concerts by international stars Adele, Rihanna and Paul McCartney.

The show, hosted by the Global BC news team, will also feature Sesame Street alumnus Bob McGrath, back for his 32nd year.

“Of all our many fundraising projects and capital campaigns, the Show of Hearts Telethon stands as the signature event,” said Variety BC executive-director Bernice Scholten. “Last year, over 1.5 million people tuned in.”

Since 1965, the children’s charity has raised over $170 million for children with special needs.

Comments

We encourage all readers to share their views on our articles and blog posts. We are committed to maintaining a lively but civil forum for discussion, so we ask you to avoid personal attacks, and please keep your comments relevant and respectful. If you encounter a comment that is abusive, click the "X" in the upper right corner of the comment box to report spam or abuse. We are using Facebook commenting. Visit our FAQ page for more information.

Saskatoon loves its Christmas lights. The Enchanted Forest draws thousands of vehicles each week. The late Bob Hinitt worked for months building his light display, which drew thousands of cars each year to Wiggins Avenue. And Scott Lambie on Clinkskill Drive has continued the tradition with more than 70,000 dancing lights (up from 50,000 last […]

When it comes to gift giving, they say it’s the thought that counts. But many are wondering if Mayor Drew Dilkens was thinking at all when he gave Olympic superstar Michael Phelps — whose battles with booze are well known — a big bottle of Canadian Club whisky.

Almost Done!

Postmedia wants to improve your reading experience as well as share the best deals and promotions from our advertisers with you. The information below will be used to optimize the content and make ads across the network more relevant to you. You can always change the information you share with us by editing your profile.

By clicking "Create Account", I hearby grant permission to Postmedia to use my account information to create my account.

I also accept and agree to be bound by Postmedia's Terms and Conditions with respect to my use of the Site and I have read and understand Postmedia's Privacy Statement. I consent to the collection, use, maintenance, and disclosure of my information in accordance with the Postmedia's Privacy Policy.

Postmedia wants to improve your reading experience as well as share the best deals and promotions from our advertisers with you. The information below will be used to optimize the content and make ads across the network more relevant to you. You can always change the information you share with us by editing your profile.

By clicking "Create Account", I hearby grant permission to Postmedia to use my account information to create my account.

I also accept and agree to be bound by Postmedia's Terms and Conditions with respect to my use of the Site and I have read and understand Postmedia's Privacy Statement. I consent to the collection, use, maintenance, and disclosure of my information in accordance with the Postmedia's Privacy Policy.